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白塞病与IgA肾病:巴西一名患者的病例报告及文献综述

Behcet's disease and IgA nephropathy: report of this association in a patient from Brazil and literature review.

作者信息

Fernandes Paula F C B S, Júnior Geraldo B Silva, Barros Fernando A S, Sousa Daniela C, Franco Luciano M, Patrocínio Régia M S V

机构信息

Serviços de Nefrologia e Reumatologia do Hospital Universitário Walter Cantídio, Brasil.

出版信息

Invest Clin. 2006 Dec;47(4):405-11.

Abstract

Behcet's disease (BD) is associated with renal involvement in about one-third of the cases and a variety of renal lesions have been reported. A 27-year-old man presented a history of recurrent oral and genital ulcers, associated with pseudofoliculitis and arthritis in his left knee. The first laboratory tests revealed: urea = 53mg/dL, creatinine = 1.8 mg/dL. The urinalysis showed leukocyturia. Initial treatment with ceftriaxone, thalidomide and prednisone was instituted. He became clinically stable, with normal renal function, but presenting hematuria and proteinuria. One year later the patient presented dark urine. The new laboratory tests showed urea=58 mg/dL, creatinine = 1.4 mg/dL, and mild proteinuria (500-1000 mg/24h). Two years later the proteinuria was 2230 mg/day. The renal biopsy showed one glomerulus with severe glomerular sclerosis, mild tubular atrophy, mild interstitial fibrosis and thickening of arterial walls. Treatment with captopril was started to decrease proteinuria. Two years later, the patient presented creatinine = 1.7 mg/dL and proteinuria = 2509 mg/day. A new renal biopsy evidenced proliferative crescentic glomerulonephritis, with diffuse granullary deposits of IgA, IgM and C3. It was instituted pulsotherapy with metilprednisolone, monthly endovenous cyclophosphamide and maintenance prednisone. The patient became clinically stable, with creatinine of 1.3 mg/dL and proteinuria of 500 mg/day. BD could be one of the various causes of secondary IgA nephritis. It is important to periodically perform renal function evaluation in patients with BD, through urinalysis and measurement of serum creatinine and its clearance, in order to detect any abnormality and provide an early adequate treatment.

摘要

白塞病(BD)约三分之一的病例会出现肾脏受累情况,且已报道有多种肾脏病变。一名27岁男性有复发性口腔和生殖器溃疡病史,伴有假性毛囊炎和左膝关节炎。首次实验室检查结果显示:尿素=53mg/dL,肌酐=1.8mg/dL。尿液分析显示白细胞尿。开始用头孢曲松、沙利度胺和泼尼松进行初始治疗。他的临床症状变得稳定,肾功能正常,但出现血尿和蛋白尿。一年后,患者尿液颜色变深。新的实验室检查显示尿素=58mg/dL,肌酐=1.4mg/dL,以及轻度蛋白尿(500 - 1000mg/24h)。两年后蛋白尿为2230mg/天。肾脏活检显示一个肾小球有严重的肾小球硬化、轻度肾小管萎缩、轻度间质纤维化和动脉壁增厚。开始用卡托普利治疗以减少蛋白尿。两年后,患者肌酐=1.7mg/dL,蛋白尿=2509mg/天。再次肾脏活检证实为增殖性新月体性肾小球肾炎,伴有IgA、IgM和C3的弥漫性颗粒状沉积。开始采用甲泼尼龙冲击疗法、每月静脉注射环磷酰胺以及维持使用泼尼松治疗。患者临床症状再次稳定,肌酐为1.3mg/dL,蛋白尿为500mg/天。白塞病可能是继发性IgA肾病的多种病因之一。对白塞病患者定期通过尿液分析、血清肌酐测定及其清除率来评估肾功能很重要,以便发现任何异常并提供早期充分治疗。

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